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KMID : 0383820090660020098
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Tuberculosis and Respiratory Diseases
2009 Volume.66 No. 2 p.98 ~ p.103
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Clinical Features of Pulmonary Langerhans Cell Histiocytosis in Korea
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Kim Cheol
Yum Ho-Kee
Kim Young-Whan
Lee Hong-Lyeol
Huh Jin-Won
Son Choon-Hee
Park Hye-Yun
Jegal Yang-Jin
Park Byung-Hoon
Park Jong-Sun
Lee Jin-Hwa
Shim Jae-Jeong
Jeong Sung-Hwan
Park Yong-Bum
Cha Seung-Ick
Chung Man-Pyo
Uh Soo-Taek
Park Choon-Sik
Kim Dong-Soon
Cho Kyung-Wook
Song Jin-Woo
Park Moo-Suk
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Abstract
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Background: Pulmonary Langerhans cell histiocytosis (PLCH) is characterized by a proliferation of Langerhans cells and this results in granulomas that involve multiple organs of the body. Because the incidence of PLCH is very
low in Korea and worldwide, collecting the clinical data of patients with PLCH nationwide is needed to determine
the clinical features of Korean patients with PLCH.
Methods: The patients with PLCH confirmed by biopsy at any body site were included and the patients should
have lung lesions present. A questionnaire that had items on the symptoms, lung function tests, the
roentgenographic findings and the treatment was collected retrospectively at a Korean ILD Research Meeting.
Results: A total of 56 cases were collected. The number of males and females was 48 and 8, respectively, and
their median age was 43 years (range: 18∼67 years). The patients were current or ex-smokers in 79% of the
cases. The most frequent symptom was coughing (39%), followed in decreasing order by dyspnea (38%), sputum
(20%) and chest pain (20%). Pneumothorax was observed in 16 (29%) patients. Lung function tests showed a
normal, restrictive, mixed or obstructive pattern in 26 (61%), 7 (16%), 7 (16%) and 3 patients (7%), respectively.
Nodular-cystic lesion was most frequently observed in 59% of the patients on HRCT. The lung lesions were located
in the middle and upper lobes in almost the cases. The median follow-up period was 90 months (range: 1∼180
months) and only two patients died during this period.
Conclusion: This study provides a national survey of the patients with PLCH during a long follow-up period.
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KEYWORD
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Langerhans, Langerhans-cell histiocytosis, Pulmonary diseases, Histiocytosis
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